Archive for May, 2014

A rare and potentially fatal lung disease sharing symptoms with so many common ailments, it can often fool the very medical specialists who are trained to recognize it.

There is no cure, and in the most severe cases — if left undetected and untreated — the patient could be dead within an average of two to three years.

About 5,000 Canadians have already been diagnosed with pulmonary hypertension (PH) — where the pulmonary arteries become scarred or closed off, leading to high blood pressure in the lungs — but as many as 10,000 might be affected by the condition, which is often misdiagnosed as asthma, allergies, or a host of other ailments.

Those who suffer from PH often spend years of frustrating trial and error before landing on the correct diagnosis, with patients often going through fruitless treatments, drug regimens and a battery of tests in the process of elimination.

And even when the correct diagnosis is made, the drugs can be costly — as much as $6,000 a month — and each comes with its own set of side effects.

Those who silently suffer will often speak of the social burdens — the isolation and depression that come with an unknown and misunderstood disease — as well as the physical and financial toll.

And despite the recent emergence of various approved treatment methods in Canada, the hospitalization rate for PH sufferers has increased 44% in the last decade.

Even more troubling, the death rate that was once in decline in males is now reversing, and the death rate for women is increased at an alarming rate.

* * *

“I finally went back to my family doctor, and I just broke down in tears,” Jo-Anne Mainwood recalls of the darkest days of her search for the mystery ailment that was stealing her breath.

“I told her, ‘I can empty my asthma medication, I can stay away from dust, and I still can’t breathe.’

“I’d go for a walk with my dog and my mom, who’s in her 70s, and I have to tell her to slow down.”

It wasn’t the pace Mainwood was accustomed to, being a Grade 8 teacher with two energetic children of her own, an active runner who could always be found enjoying the great outdoors.

It was her birthday, and Mainwood had invited friends to run the aerial course at the Lefleche Caves near Val-Des-Monts.

That’s the first moment — some nine years ago now — Mainwood can recall being embarrassed to be lagging so far behind.

“I kept running completely out of air, having to walk three feet and then having to bend down and try to catch my breath. It was horrible for somebody who’d always been doing outdoorsy stuff. I just couldn’t breathe and I couldn’t keep up.

“So I saw my doctor, and I was told, ‘Well you’ve had a couple of kids, you’ve put on a few pounds, maybe just do a bit more exercise and see if getting back into shape helps you.’

“So that was the first thing, and I joined a gym and I’d try to exercise, but I’d feel gross and faint. I remember being on the treadmill, and I’d look to the person beside me running like mad, and I’m barely walking and my heart beat is going just as fast as theirs.”

She went back to her doctor, and tested positive for asthma.

“So I got on the (asthma inhalers) but I was still struggling,” says Mainwood. “I’d go up a flight of stairs and I’d still be completely winded.”

Back to the doctor, where yet another best guess pointed to a dust allergy as the culprit.

“It was so frustrating,” she says as she was finally sent for a series of tests, including a stress test that uncovered a slightly irregular heartbeat, and a nuclear isotope test that identified what doctors thought were blood clots in her lungs.

“That’s when I knew something was up, when they asked me to stay in hospital,” says Mainwood.

She was taught how to administer her own blood thinners to keep the clots under control, which would have been valuable knowledge had the diagnosis been the correct one.

Five years would pass from the time Mainwood first noticed her shortness of breath to the point where PH was properly identified, through a procedure called a right heart catheterization, which takes a direct measurement of the blood pressure in pulmonary arteries.

“The hard part was that while I was showing symptoms of asthma (early on), my respirologist is an expert on pulmonary hypertension, and he was the one who at first misdiagnosed it as asthma,” says Mainwood, illustrating the challenge too often facing both sufferers and the medical community.

“And the problem is, the longer you go without treatment, the more damage is done — the more sickening and scarring and hardening of the pulmonary arteries takes place, and the harder it is on your heart.

“So you’re getting worse and worse as you’re waiting for a diagnosis.”

* * *

But in the absence of a cure, coming to the correct conclusion on the diagnosis is cold comfort.

“Even when you know what you have,” says fellow PH sufferer Charlene Smith, 74, “you don’t know how it’s going to affect you in a lifetime point-of-view. So I just wake up in the morning and live one day at a time.”

Smith, who has been dealing with PH for seven years, recently started a support group, where about 17 local PH sufferers, their family members and their caretakers can share their experiences.

“It really hit me when I started meeting other PH patients at a conference,” says Smith. “But for the first couple of years I felt very isolated. They were very good to me at the Ottawa Heart Institute, but it’s not the same as having someone with you who knows what you’re experiencing.”

In a recent survey undertaken by the Pulmonary Hypertension Association of Canada — the first study of its kind, and only coming in late 2013 — it was noted that seven in 10 patients experienced “social isolation and relationship issues,” attributed primarily to the general lack of knowledge, or “invisibility” of the disease.

That started early in the journey for Smith, who moved out to Ottawa from Vancouver saddled with a pair of oxygen tanks, which doctors had mistakenly told her she’d rely upon for the rest of her life to treat yet another misdiagnosed condition — in this case, cardiac obstructive pulmonary disease.

She, too, eventually identified PH as the invisible menace, but that was only the beginning of a new cycle, with different drugs prescribed to combat the side effects that would emerge from the last batch of medication.

“It’s like a vicious circle,” Smith says. “And facing life, it’s day by day. There are days when you think you can’t make it through the day.

“I accept it now, but for me, it took quite awhile to accept it.”

* * *

For Mainwood, she too arrived at acceptance, but not before arriving at a number of conflicting emotions along the way.

“I thought I was going crazy — it was depression, I was lethargic, I just wanted to come home from work and sleep,” she recalls.

“Even after being diagnosed it was hard, because I was so tired. My cardiac output is half of what a normal persons’ would be.”

Mainwood eventually began to cope, with little adjustments like having her second-floor classroom shifted to the main floor to avoid stairs, or having her husband take over the “lion’s share” of domestic duties.

Even then, Mainwood bristles at the memory of a dinner guest remarking that she must be some kind of “princess” to have such a doting and attentive partner.

While she’d seethe through the occasional comment, soon she’d come to crave any social interaction at all.

“I was a foster parent, I have my own two kids, I’m teaching Grade 8 and putting on school productions and events and trying to keep up, but it’s just getting harder, so I ended up not having much of a social life,” says Mainwood.

“It was all I could manage. And even if I did manage to go out with friends, I’d be so tired I couldn’t follow the conversation, and I’d just want to put my head down and sleep.”

Then there was the drug regimen, which would run between $5,000 and $6,000 each month, paid up front and later partially covered by insurance.

“But at first it was so stressful,” she says, “Because if you didn’t get reimbursed after two months you’re already $12,000 in debt. So you can’t have much of a social life anyway, because you simply can’t afford it.”

Other challenges would prove even more difficult.

“There is no cure, and that was hard, too. Because then I wanted to know how long do I have to live. You have to process that this is permanent.

“I can breathe better and I can do things I couldn’t before, but I keep waiting for the other shoe to drop, when the drugs don’t do the job any more.”

Even with the drugs — part of the treatment requires three shots a day of the same active ingredient in Viagra — there are side effects that come in the form of “almost debilitating” body aches and liver problems.

Mainwood was rattled when she recently started coughing up blood, confiding once again in her doctor.

“He told me, ‘You know, I know you’re upset about this and you’re worried, but to put this in perspective, if you didn’t get the treatment, you’d be dead now.’

“So, living with the disease, I’m thankful it was diagnosed. Every day is a gift, because without being treated, I’d be dead.”

PH is a chronic condition defined by the arteries of the lungs becoming closed off or scarred, causing high blood pressure in the lungs. As the disease progresses, the heart can become enlarged, with potentially fatal consequences.

PH can strike at any time and has no regard for race or age.

Physical symptoms can include:

Breathlessness or shortness of breath, especially with activity.
Fainting and/or feeling tired all the time.
Lightheadedness, especially when climbing stairs or standing up.
Swollen ankles, legs, or abdomen.
Chest pain, especially during physical activity.
PH is often not diagnosed correctly, because early symptoms are subtle and can be confused with those of many other conditions.

Many people suffer from PH without knowing it, and the chronic condition can be fatal if left untreated.

“Invisibility” of the disease, delays in diagnosis and physical, social and financial burdens among top challenges to be addressed

VANCOUVER, May 5, 2014 /CNW/ – Results released today from the Pulmonary Hypertension Association (PHA) of Canada’s Patient and Caregiver Survey reveal that Canadians with pulmonary hypertension (PH) are struggling to live with the social, physical and financial burdens the disease places on their lives. Respondents list social isolation, lack of disease awareness and both physical and financial burdens as the top challenges that need to be addressed.

Conducted online by Harris Poll on behalf of PHA Canada in late 2013, the survey of 179 respondents (118 patients and 61 caregivers) is the first of its kind conducted in Canada to measure the impact of PH – a rare, debilitating, progressive and potentially fatal lung disease affecting as many as 10,000 Canadians.

“We are grateful to the many Canadians who shared their experiences of living with PH through this important research,” says Angie Knott, National Manager, Pulmonary Hypertension Association of Canada. “Their feedback confirms that too many Canadians are still struggling to live and cope with this rare and fatal disease and they have provided us with valuable insights into what changes are needed most across the country to improve lives.”

Social isolation and relationship issues – for patients and caregivers alike

Every day, more than 70% of patients surveyed and more than 61%* of caregivers surveyed experience social isolation and relationship issues, which they attribute to the “invisibility” of the disease, according to the PHA Canada Patient and Caregiver Survey. The general lack of awareness and understanding amongst their friends, colleagues and the general public is the primary cause of these feelings of social isolation.

In addition, the physical burden of the disease places many social limitations on those affected by it, with seven in 10 patients surveyed reporting they are unable to play with their children/grandchildren or even help them with their homework. Caregivers are also impacted as those surveyed spend more than half* of their time on activities related to caring for their relative with PH.

“Every day has become a challenge since my PH diagnosis,” said Loretta Chu, of Toronto, Ontario, who has lived with PH for eight years. “When you are dealing with symptoms that others cannot visibly see or relate to, people are quick to misunderstand and even judge.”

Physical limitations and reduced quality of life

The PHA Canada Patient and Caregiver Survey also reveals that 85% of patients surveyed experience mild to severe symptoms or limitations with every-day activities such as walking and climbing stairs. More than 40% of patients surveyed report frequently suffering from fatigue, low energy and breathlessness, while another 37% suffer from sleep disorders.

These physical symptoms make it extremely difficult for patients to continue living healthy, happy lives and force many, along with their caregivers, to make dramatic changes to their lifestyle, including their professional and personal relationships. Even with current treatments, one-third of patients surveyed feel there are insufficient medication options to optimally manage their PH.

Opportunity costs – impact on employment and income

Due to the physical limitations of the disease, patients also find it very difficult to maintain their employment, with nearly 60% of those surveyed reporting they are either no longer able to work at all or have partially stopped working due to their PH. There is a similar impact on caregivers too, with close to 40%* of caregivers forced to make employment changes to care for someone with PH.

This inability to work carries enormous psychological and social challenges, and also has a tremendous impact on the financial stability of those affected by PH, many of whom are in their prime earning years. Of those surveyed, nearly 90% of patients say that their income has decreased by more than 25% due to their PH-induced change in employment, with nearly half saying their income has reduced by 50% or more.

“The symptoms of PH, like chronic fatigue and shortness of breath, make it extremely difficult to carry out every-day activities that most Canadians take for granted,” said Loretta Chu, who also leads a support group for PH patients in Toronto. “This obviously makes it very hard to maintain one’s employment which brings not only financial income and stability but also a sense of purpose, achievement and social interaction. When all of this is taken from someone, the burden is tremendous.”

The killer waiting game – delays in proper diagnosis

Citing several reasons, including a lack of recognition of symptoms among patients and their primary healthcare providers, the PHA Canada Patient and Caregiver Survey reveals that surveyed patients waited an average of three years for proper diagnosis. While more than half of patients surveyed state that their family doctor did not initially recognize the symptoms of PH and refer them to the proper specialist, patients themselves suffered an average of 31 and 44 months with breathlessness/dyspnea and tiredness/fatigue respectively before even seeking medical attention.

“This survey has uncovered an alarming lack of awareness of PH and its symptoms. Like many rare and fatal diseases, early diagnosis and access to effective treatment can make an enormous impact on outcomes and quality of life. We now know that we must do a better job of educating both doctors and the general public in PH to help improve, and save lives,” said Dr. Sanjay Mehta, MD, FRCPC, FCCP, Professor of Medicine at Western University, Director of the Southwest Ontario Pulmonary Hypertension Clinic at the London Health Sciences Center in London, Ontario and Chair of the Pulmonary Hypertension Association of Canada.

*Caution – small base (n<100). Results should be interpreted as qualitative, or directional, in nature.

About Pulmonary Hypertension (PH)

Pulmonary hypertension (or PH) is a rare disease affecting the arteries of the lungs. When an individual has PH, the arteries of their lungs become narrowed and scarred. This can result in almost complete closing of the arteries, which can lead to heart failure. Some symptoms of PH are shortness of breath; bluish hands, feet and lips; swelling of hands and feet; light-headedness and dizziness; chest pain; exhaustion and fainting. Because it shares many symptoms with other diseases, PH is often misdiagnosed. PH is a terminal disease for which there is currently no cure.

About the Survey

Harris Poll conducted this online Burden of Illness survey across Canada on behalf of PHA Canada between September 12 and October 25, 2013, among a total of 179 respondents (118 patients and 61 caregivers). Statistical comparisons were conducted at the 95% level of confidence between groups where base sizes allowed. The average age of the patients and caregivers who completed the survey was 51 and 47 respectively. Respondents completed online questionnaires developed by PHA Canada with the guidance of a steering committee including PH doctors, nurses, patients and caregivers. The survey was conducted by Harris Interactive and funded by Actelion Pharmaceuticals Canada Inc. through an unrestricted educational grant to PHA Canada. It is the first survey conducted in Canada to measure the impact of PH on patients and their caregivers, and has allowed a deeper understanding of PH and the physical, psychological and emotional burden the disease has on the lives of those affected by it.

Please visit www.phacanada.ca for more information and to locate your community support organization for pulmonary arterial hypertension.

About the Pulmonary Hypertension Association of Canada (PHA Canada)
The Pulmonary Hypertension Association of Canada (PHA Canada) is a national, registered charity. Our mission is to empower the Canadian pulmonary hypertension community through awareness, advocacy, education, research and patient support. PHA Canada is working to end the invisibility and isolation often experienced by those living with this life-changing disease. To learn more about PHA Canada and pulmonary hypertension, please visit www.phacanada.ca.

BC Place, 777 Pacific Blvd, Vancouver, BC V6B 4Y8
BC Place is the largest sports, exhibition and entertainment venue of its kind in British Columbia, hosting the province’s most notable events including the Opening and Closing Ceremonies of the Vancouver 2010 Olympic Winter Games.

Every evening beginning at dusk, the Falls are lit in the colours of the rainbow. Don’t miss the unmatched beauty of the Falls at night! Illumination of the Falls has been financed and operated by The Niagara Falls Illumination Board since 1925.

Niagara Falls will light up purple for #WorldPHDay! Special colour illuminations are offered at 9:00 p.m. and 10:00 p.m. (15 minute duration), subject to availability. Both Falls are illuminated. Illumination times are approximate and subject to change according to light conditions.

The Peace Bridge Will also light up purple for #WorldPHDay!
The Peace Bridge is an international bridge between Canada and the United States at the east end of Lake Erie at the source of the Niagara River, about 20 kilometres upriver of Niagara Falls.